Surgery Promises Relief for Dystonia

When Peter Cohen entered a public swimming pool recently, he was greeted with several rounds of applause. Cohen is not a person whom one would normally expect to be met with such a reception; he has never been an Olympian, nor has he ever held public office.

Merely attempting to move about freely in a pool was regarded as heroic, because, as is widely known throughout his home community of South Portland, Maine, Cohen has battled dystonia, a debilitating nerve disease that causes unwanted motor movements.

Because of a new treatment, Cohen’s condition has improved considerably. For the first time in decades, he is able to perform many normal activities.

The treatment is called deep brain stimulation, or, officially, “Activa Dystonia Therapy.” Manufactured by Medtronics, it was developed for people with Parkinson’s.

For Cohen, the treatment is welcome. Now 39 years old, he was diagnosed with dystonia as a teenager after experiencing relatively minor symptoms, such as difficulty making fine movements with his right hand.

The disease progressed quickly. In college and law school, Cohen had to use a typewriter to take exams. At times, he had to sit on his right arm to suppress its incessant jerking. The act of walking small distances became arduous, as his right leg would flail unexpectedly. His neck would sporadically turn sideways and downward. Despite these obstacles, Cohen passed the bar and began a career as an attorney.

Gradually, his symptoms worsened. Much to his dismay, he had to stop practicing law.

“I was bent over, I was lying down,” he said. Dystonia had truly taken over his life.

Cohen heard of deep brain stimulation at a dystonia conference he attended in 2002. The science of deep brain stimulation is not new, having been developed for Alzheimer’s patients. Yet doctors were unsure if the same method could be applied to dystonia sufferers. Cohen decided to undergo the new treatment. He had little to lose, and, in his words, “I had great faith in the doctors.”

Cohen underwent an operation at Emory University Hospital in Atlanta on February, 17, 2002. In a four-hour procedure, Dr. Jerrold Vitek and Dr. Aviva Abosch drilled a hole into the globus pallidum area of his brain, from which they led wires underneath his scalp and down the back of his neck to a pacemaker in his chest.

Cohen lay awake during the procedure. Only a few centimeters separate the globus pallidum from other sensitive areas of the brain. It is believed that dystonia originates in that area. Although the doctors mapped Cohen’s brain extensively before the operation and were thus aware of his brain’s boundaries, they had to make sure that they were affecting only that area and not other nearby regions.

February 17 would not be the only tiresome day for Cohen. During those four hours, the doctors had operated on only one side of his brain. During the next several months, he would have to return to Atlanta three times, and he must return several more times for follow-ups.

The surgeries did not proceed without incident. Abosch recalled the difficulty of placing the frame that was intended to secure the twitchy Cohen during surgery on his body.

“That’s the nature of the disease,” she said. For a few weeks after the second surgery, Cohen struggled with horrendous headaches that Vitek believes were caused by air that snuck into the brain during surgery.

In total, his deep brain stimulation cost approximately $100,000, but fortunately for Cohen, Medicare picked up the bill.

“The issue is trying to get it covered by most insurance providers because it’s still labeled as ‘experimental,’ and insurance companies don’t cover what is deemed to be experimental,” Abosch said.

How deep brain stimulation works remains somewhat of a mystery. The doctors said they redirected energy from the section of the brain that was causing the dystonia. Yet an exact medical explanation for why the surgery works has yet to be articulated. For now, doctors and patients are content to proceed as long as procedure promises relief

“I’m starting to see some benefits. I can now sit up straight, where before I had to slouch. My walking has gotten better,” Cohen said. “It feels amazing to get my independence back.”

Robert McAlister, the executive director of the Dystonia Medical Research Foundation, said: “We’ve seen so many potential therapies that have come and gone, but this one looks extremely exciting.”

“I base that comment on a relatively small number of patients that we know about that have experienced the surgery,” he said. “The results that we know about have been almost miraculous.”

Physicians traditionally use the Burke-Fahn-Marsden dystonia scale as a barometer of how the disease is affecting the muscles. Doctors who have performed deep brain stimulation on dystonia patients claim to have witnessed 65% to 90% increases on the BFM scale, which they say is an excellent measure of improvement.

A handful of hospitals are offering the new therapy. Beth Israel Medical Center in New York City has helped people of many different ages and both genders, according to the dystonia foundation.

“It’s not a cure,” said Dr. Michele Tagliati of Beth Israel Medical Center. “It’s a symptomatic treatment, but for dystonia, there are few medical treatments, and the pharmacological treatment is full of side effects.”

In August 2002, Cohen was married. Unfortunately for the newlyweds, dystonia made a honeymoon impossible. Now, thanks to deep brain stimulation, the couple is planning one for later this year.

“I’m sure it’s been very hard on my wife, as it has been hard on me,” he said. “I want to be able to do stuff with her. I want to be able to go outside, go to the beach or even have dinner.”

Now that his condition has improved, Cohen is focusing on other people’s suffering, serving as a board member of the Dystonia Medical Research Foundation. In the worst days of his affliction, he said, he never lost hope: “I just had faith everything would be okay.”

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